Papillary tumour of the pineal region

Department of Neurosurgery, Addenbrooke's Hospital, University of Cambridge, CB2 2QQ, UK.
British Journal of Neurosurgery (Impact Factor: 0.95). 03/2008; 22(1):116-20. DOI: 10.1080/02688690701583794
Source: PubMed

ABSTRACT Papillary tumour of the pineal region (PTPR) is a relatively new and rare pathological entity, which appears to run a spectrum of clinical courses. We add another case with detailed description of the clinical course documented with serial imaging over the total of 7 years. In accordance with previous reports we recommend total surgical resection with subsequent focal radiotherapy. Clinical and radiological follow up of the entire cerebrospinal axis is mandatory.

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    ABSTRACT: Papillary tumor of the pineal region (PTPR) is a recently described distinct clinicopathological entity. The purpose of this case report is to increase the knowledge of its neuroradiological findings and natural history by describing the long-term clinical and neuroradiological follow-up of a PTPR occurring in a 56-year-old Italian male patient. At magnetic resonance imaging (MRI) obtained at diagnosis, the lesion showed a subtle high signal intensity on T1-weighted imaging. Twenty-nine months later, MRI showed clearcut enlargement of the lesion, which had only a small area of high signal intensity on T1-weighted images, and a minimum apparent diffusion coefficient of 0.854 x 10(-3) mm(2)/s. Treatment included surgery followed by irradiation. Three-month MRI follow-up did not show disease relapse.
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    ABSTRACT: We report a boy who in 1994, at the age of 11, presented with headache and vomiting. The fi nal diagnosis was papillary tumor of the pineal region (PTPR). Magnetic resonance imaging (MRI) revealed a heterogeneous mass. Hydrocephalus was addressed by immediate ventricle drainage; subsequently, we attempted tumor removal. As the intraoperative diagnosis of the hemorrhagic tumor was primitive neuroectodermal tumor (PNET), we did not proceed to total removal. After the delivery of radiotherapy (50.4 Gy) and one course of Nimustine hydrochloride (ACNU) chemotherapy, the residual tumor was completely resected. The diagnosis at that time (1994) was papillary pineocytoma. He was followed on an outpatient basis for 15 years and remained free of recurrence. This type of tumor was later proposed to represent a new distinct tumor subtype, papillary tumor of the pineal region (PTPR). Our data indicate that our patient's tumor should be included in this category.
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